title: Clinical and pathologic features of neuronal ceroid-lipofuscinosis in a ferret (Mustela putorius furo).
abstract: Clinical and pathologic features of neuronal ceroid-lipofuscinosis in a 4-month-old ferret are reported. Clinical signs including neurological symptoms appeared at 3 months of age and progressed rapidly. By magnetic resonance imaging, severe cerebral atrophy was recognized. Histopathologically, there was severe neuronal loss and diffuse astrogliosis with macrophage accumulations; lesions were found predominantly in the cerebral cortex. Intracytoplasmic pigments were observed in surviving neurons and macrophages throughout the brain. The pigments were intensely positive for periodic acid-Schiff, Luxol fast blue, and Sudan black B and exhibited a green autofluorescence. Electron microscopic examination revealed the accumulation of electron-dense granular material within lysosomes of neurons and macrophages. Immunohistochemically, a large number of saposin-positive granules accumulated in the neuronal cells, astrocytes, and macrophages of the lesions, but significant immunoreactivity for subunit c of mitochondrial adenosine triphosphate synthase was not observed. Based on these findings, the animal was diagnosed as affected by neuronal ceroid-lipofuscinosis.
authors.affiliation: Faculty of Agriculture, Miyazaki University, Miyazaki, Japan.
authors.forename: K
authors.initials: K
authors.lastname: Nibe
authors.forename: Y
authors.initials: Y
authors.lastname: Miwa
authors.forename: S
authors.initials: S
authors.lastname: Matsunaga
authors.forename: J K
authors.initials: JK
authors.lastname: Chambers
authors.forename: K
authors.initials: K
authors.lastname: Uetsuka
authors.forename: H
authors.initials: H
authors.lastname: Nakayama
authors.forename: K
authors.initials: K
authors.lastname: Uchida
mesh_terms: D000818:Animals; D015415:Biomarkers / Q000378:metabolism; D001921:Brain / Q000473:pathology* / Q000648:ultrastructure; D002540:Cerebral Cortex / Q000473:pathology / Q000648:ultrastructure; D037901:Euthanasia, Animal; D005260:Female; D005289:Ferrets; D007150:Immunohistochemistry / Q000662:veterinary; D008264:Macrophages / Q000473:pathology; D025261:Mitochondrial Proton-Translocating ATPases / Q000378:metabolism; D009472:Neuronal Ceroid-Lipofuscinoses / Q000473:pathology / Q000662:veterinary*; D009474:Neurons / Q000473:pathology; D011817:Rabbits; D049231:Saposins / Q000378:metabolism
chemical_list: D015415:Biomarkers; D049231:Saposins; C103607:mitochondrial ATPase subunit c; D025261:Mitochondrial Proton-Translocating ATPases